A registry of patients with severe deficiency of alpha1-antitrypsin: Design and methods

M. D. Schluchter; A. F. Barker; R. G. Crystal; R. A. Robbins; J. M. Stocks; J. K. Stoller; M. C. Wu; Z. Bengali; C. E. Vreim; A. Rothgeb; G. L. Snider; K. Detre; H. Y. Reynolds; M. S. Tockman; J. Wittes; A. S. Buist; B. Burrows; A. Cohen; R. J. Fallat

doi:10.1378/chest.106.4.1223

A registry of patients with severe deficiency of alpha₁-antitrypsin: Design and methods

M. D. Schluchter, A. F. Barker, R. G. Crystal, R. A. Robbins, J. M. Stocks, J. K. Stoller, M. C. Wu, Z. Bengali, C. E. Vreim, A. Rothgeb, G. L. Snider, K. Detre, H. Y. Reynolds, M. S. Tockman, J. Wittes, A. S. Buist, B. Burrows, A. Cohen, R. J. Fallat

Research output: Contribution to journal › Article › peer-review

53 Scopus citations

Abstract

The Registry of Patients with Severe Deficiency of Alpha₁-Antitrypsin (AIAT) is a multicenter natural history study, with 37 participating clinical centers in the United States (36 centers) and Canada (1 center). The study has enrolled 1,129 individuals aged ≥18 years with severe deficiency of AIAT (serum level ≤11 μM), and will follow them longitudinally for up to 7 years, characterizing the clinical course of the disease, regardless of whether they are receiving augmentation therapy. Primary outcomes of interest are the yearly decline in FEV₁ and mortality. This article describes the design and structure of the Registry.

Original language	English (US)
Pages (from-to)	1223-1232
Number of pages	10
Journal	CHEST
Volume	106
Issue number	4
DOIs	https://doi.org/10.1378/chest.106.4.1223
State	Published - 1994
Externally published	Yes

Keywords

alpha-antitrypsin
augmentation therapy
decline in lung function
design of studies
emphysema
longitudinal studies
registries
replacement therapy

ASJC Scopus subject areas

Pulmonary and Respiratory Medicine
Critical Care and Intensive Care Medicine
Cardiology and Cardiovascular Medicine

Access to Document

10.1378/chest.106.4.1223

Cite this

Schluchter, M. D., Barker, A. F., Crystal, R. G., Robbins, R. A., Stocks, J. M., Stoller, J. K., Wu, M. C., Bengali, Z., Vreim, C. E., Rothgeb, A., Snider, G. L., Detre, K., Reynolds, H. Y., Tockman, M. S., Wittes, J., Buist, A. S., Burrows, B., Cohen, A., & Fallat, R. J. (1994). A registry of patients with severe deficiency of alpha₁-antitrypsin: Design and methods. CHEST, 106(4), 1223-1232. https://doi.org/10.1378/chest.106.4.1223

Schluchter, MD, Barker, AF, Crystal, RG, Robbins, RA, Stocks, JM, Stoller, JK, Wu, MC, Bengali, Z, Vreim, CE, Rothgeb, A, Snider, GL, Detre, K, Reynolds, HY, Tockman, MS, Wittes, J, Buist, AS, Burrows, B, Cohen, A & Fallat, RJ 1994, 'A registry of patients with severe deficiency of alpha₁-antitrypsin: Design and methods', CHEST, vol. 106, no. 4, pp. 1223-1232. https://doi.org/10.1378/chest.106.4.1223

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abstract = "The Registry of Patients with Severe Deficiency of Alpha1-Antitrypsin (AIAT) is a multicenter natural history study, with 37 participating clinical centers in the United States (36 centers) and Canada (1 center). The study has enrolled 1,129 individuals aged ≥18 years with severe deficiency of AIAT (serum level ≤11 μM), and will follow them longitudinally for up to 7 years, characterizing the clinical course of the disease, regardless of whether they are receiving augmentation therapy. Primary outcomes of interest are the yearly decline in FEV1 and mortality. This article describes the design and structure of the Registry.",

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author = "Schluchter, {M. D.} and Barker, {A. F.} and Crystal, {R. G.} and Robbins, {R. A.} and Stocks, {J. M.} and Stoller, {J. K.} and Wu, {M. C.} and Z. Bengali and Vreim, {C. E.} and A. Rothgeb and Snider, {G. L.} and K. Detre and Reynolds, {H. Y.} and Tockman, {M. S.} and J. Wittes and Buist, {A. S.} and B. Burrows and A. Cohen and Fallat, {R. J.}",

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AU - Detre, K.

AU - Reynolds, H. Y.

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AU - Cohen, A.

AU - Fallat, R. J.

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